Beyond a broken heart: circulatory dysfunction in the failing Fontan

Area of Science:

• Cardiology
• Hepatology
• Physiology

Background:

• Ventricular dysfunction is a known factor in univentricular heart outcomes.
• A significant number of adult Fontan patients face mortality or heart transplantation with preserved ventricular function.

Purpose of the Study:

• To broaden the understanding of circulatory dysfunction in adult Fontan patients.
• To focus on the pathophysiology of multi-organ involvement, particularly hepatic dysfunction and portal hypertension.

Main Methods:

• Review of existing literature on hepatic perfusion, liver histopathology, and liver disease in Fontan patients.
• Comparison of postsinusoidal portal hypertension in Fontan physiology with adult cirrhotic disease.
• Delineation of the systemic, pulmonary, renal, and cardiac effects of portal hypertensive physiology.

Main Results:

• Clinical deterioration in Fontan patients necessitates a view beyond cardiac function.
• Hepatic dysfunction and portal hypertension are key contributors to multi-organ involvement.
• Portal hypertension significantly impacts systemic and pulmonary vasculature, kidneys, and the heart.

Conclusions:

• A comprehensive understanding of circulatory dysfunction in adult Fontan patients requires considering multi-organ interactions.
• Hepatic involvement and portal hypertension are critical factors influencing long-term outcomes.
• These pathophysiological changes have direct implications for managing adult Fontan patients.